OBJECTIVE: The Authors report their experience in diagnosis and treatment of one case of mesenteric cystic lymphangioma; recent international literature review. EXPERIMENTAL DESIGN: Complete clinical report. Diagnostic, clinical and prognostic indication, evaluation of effectiveness of radical surgical treatment and follow-up. SETTING: Operative Unit of General and Thoracic Surgery. University “Paolo Giaccone” of Palermo. INTERVENTION: Radical surgical treatment, according to international guide-lines. RESULTS: Complete recovery with “restitutio ad integrum”. No relapse were recorded at short follow up. CONCLUSIONS: Even when asymptomatic and discovered incidentally, LCM must be treated surgically because of the potential to grow, invade vital structures, and develop life-threatening complications. The best and only radical treatment is the total removal of the lesions with microscopically clear borders, when it is available.